Angiosarcoma of the heart predominantly involving the pericardium: A clinicopathologic study with electron-microscopic and immunohistochemical correlation

A case of angiosarcoma of the heart with predominant involvement of the pericardium and diffuse bilateral lymphangitic spread to both lungs is presented. The patient was a 32-year-old man admitted for cough, dyspnea, and intermittent hemoptysis. Chest x-ray and computed tomography scans revealed bilateral pulmonary infiltrates and pericardial effusion. An echocardiogram showed a large oval mass overlying and compressing the right atrium and ventricle. At autopsy, multiple hemorrhagic tumor nodules were present in the pericardial sac, and an 8-cm hemorrhagic tumor mass was found overlying and compressing the right atrial appendage and infiltrating the heart muscle. Histological, immunohistochemical, and electron-microscopic examination revealed the features of angiosarcoma. The predominant pericardial location observed in this case was a source of difficulty both in the clinical and pathological differential diagnosis and raised the question of possible primary pericardial angiosarcoma with secondary involvement of the heart. A review of the literature on this topic is presented.